Langford C A, Sneller M C
Immunologic Diseases Section, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Curr Opin Rheumatol. 1997 Jan;9(1):26-30. doi: 10.1097/00002281-199701000-00005.
With the opportunity for long-term follow-up, it has been appreciated that disease severity may vary considerably in patients with Wegener's granulomatosis, polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Although combined therapy with cyclophosphamide and glucocorticoids continues to be the foundation of treatment, concerns have increased about the toxicities of this regimen. As illustrated by studies on Wegener's-related subglottic stenosis and endobronchial involvement, it has also become apparent that some disease manifestations may not respond to this therapy. Recent therapeutic investigations have therefore focused on identifying approaches that are less toxic and that are based on anatomic involvement and disease severity. The study of polyarteritis nodosa has additionally been affected by proposed changes in classification that would make this condition a separate entity from microscopic polyangiitis. As progress is made in defining the clinical significance of this nomenclature, the prognostic factors in these patients have also been examined, which may be helpful in guiding therapeutic decisions.
随着长期随访机会的出现，人们认识到韦格纳肉芽肿、结节性多动脉炎、显微镜下多血管炎和变应性肉芽肿性血管炎患者的疾病严重程度可能有很大差异。虽然环磷酰胺和糖皮质激素联合治疗仍然是治疗的基础，但对该方案毒性的担忧有所增加。正如关于韦格纳相关声门下狭窄和支气管内受累的研究所表明的那样，一些疾病表现可能对这种治疗没有反应，这一点也变得很明显。因此，最近的治疗研究集中在确定毒性较小且基于解剖学受累情况和疾病严重程度的方法上。结节性多动脉炎的研究还受到分类提议变化的影响，这些变化将使这种疾病与显微镜下多血管炎成为不同的实体。随着在确定这种命名法的临床意义方面取得进展，这些患者的预后因素也得到了研究，这可能有助于指导治疗决策。
